Recent advances in understanding the pathophysiology of primary T cell immunodeficiencies

Alain Fischer

doi:10.1016/j.molmed.2015.04.002

Recent advances in understanding the pathophysiology of primary T cell immunodeficiencies

Trends Mol Med. 2015 Jul;21(7):408-16. doi: 10.1016/j.molmed.2015.04.002. Epub 2015 May 4.

Author

Alain Fischer¹

Affiliation

¹ Paris Descartes-Sorbonne Paris Cité University, Imagine Institute, Paris, France; Immunology and Pediatric Hematology Department, Assistance Publique-Hôpitaux de Paris, Paris, France; Institut National de la Santé et de la Recherche Médicale (INSERM) Unité Mixte de Recherche 1163, Paris, France; Collège de France, Paris, France. Electronic address: alain.fischer@inserm.fr.

PMID: 25953674
DOI: 10.1016/j.molmed.2015.04.002

Abstract

Primary immunodeficiencies (PIDs) constitute a large group of rare disorders that affect the function of the immune system. These deficiencies are usually associated with inability to prevent infections but can also result in allergy, inflammation, autoimmunity, and cancer. The recent characterization of many different monogenic defects has shed light on a range of non-redundant differentiation, effector, and regulatory functions of immune cells. The present review focuses on PIDs that affect adaptive immunity in general and T lymphocytes in particular. The analysis of PIDs is now opening up new pathways towards targeted therapies.

Keywords: T lymphocytes; autoimmunity; inflammation; innate immunity; primary immunodeficiencies.

Publication types

Review

MeSH terms

Animals
Autoimmunity / immunology
Humans
Immunity, Innate / immunology
Immunologic Deficiency Syndromes / immunology*
Primary Immunodeficiency Diseases
T-Lymphocytes / immunology

Supplementary concepts

T cell immunodeficiency primary